Retiform hemangioendothelioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult
- Status
- active
A rare vascular tumor characterized by a slowly growing lesion with predominant involvement of the skin and subcutaneous tissue of the distal extremities. Distinctive arborizing blood vessels lined by endothelial cells with characteristic hobnail morphology are a typical feature. Local recurrences are frequent unless wide local excision is performed, while metastasis is rare.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Retiform hemangioendothelioma | |
| fr | Hémangioendothéliome rétiforme | |
| de | Retiformes Hämangioendotheliom | |
| es | Hemangioendotelioma retiforme | |
| it | Emangioendotelioma retiforme | |
| nl | Retiform hemangio-endothelioom | |
| pt | Hemangioendotelioma retiforme | |
| pl | Naczyniakośródbłoniak sieciowaty | |
| cs | Retiformní hemangioendoteliom | |
| uk | Ретиформна гемангіоендотеліома | |
| zh | 网状血管内皮瘤 | |
| tr | Retiform hemanjioendotelyoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D48.1 | NTBT | attributed | validated |
| MONDO | 0018713 | E | validated | |
| UMLS | C1304512 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:673473Borderline vascular tumor
rare developmental anomalies during embryogenesis; rare circulatory system diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 32 cases | Worldwide | Case(s) | Validated | 25812740[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_25812740[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/458763
- This record as JSON: /data/d/458763.json